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Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease

Ewing sarcoma (ES) is the second most common malignant bone tumor in children and has also been described in adults with highly aggressive behavior. ES belongs to the small round blue cell tumor family and presents the distinctive translocation of FET-ETS family genes (85% with EWSR1), generating ge...

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Autores principales: Valeri, Marina, Dore, Leocadia, Rudini, Noemi, Cieri, Miriam, Elefante, Grazia Maria, Saita, Alberto, Bertuzzi, Alexia, Colombo, Piergiuseppe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9851605/
https://www.ncbi.nlm.nih.gov/pubmed/36686730
http://dx.doi.org/10.3389/fonc.2022.1070838
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author Valeri, Marina
Dore, Leocadia
Rudini, Noemi
Cieri, Miriam
Elefante, Grazia Maria
Saita, Alberto
Bertuzzi, Alexia
Colombo, Piergiuseppe
author_facet Valeri, Marina
Dore, Leocadia
Rudini, Noemi
Cieri, Miriam
Elefante, Grazia Maria
Saita, Alberto
Bertuzzi, Alexia
Colombo, Piergiuseppe
author_sort Valeri, Marina
collection PubMed
description Ewing sarcoma (ES) is the second most common malignant bone tumor in children and has also been described in adults with highly aggressive behavior. ES belongs to the small round blue cell tumor family and presents the distinctive translocation of FET-ETS family genes (85% with EWSR1), generating gene fusions. Extraskeletal ES mainly occurs in soft tissues; the urogenital tract is rarely affected, and ureteral localization is an exceptional event with only 4 cases described in the literature. Here we report the first Italian case of primary ES of the ureter, a 24-year-old young man with lower back pain and a narrowed left ureteral lumen on CT scan. ES of the urogenital tract is an almost unique condition with a nonspecific clinical presentation and a challenging diagnosis for pathologists. We encourage awareness of these exceptional events in the differential diagnosis of ureteral lesions in young patients.
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spelling pubmed-98516052023-01-20 Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease Valeri, Marina Dore, Leocadia Rudini, Noemi Cieri, Miriam Elefante, Grazia Maria Saita, Alberto Bertuzzi, Alexia Colombo, Piergiuseppe Front Oncol Oncology Ewing sarcoma (ES) is the second most common malignant bone tumor in children and has also been described in adults with highly aggressive behavior. ES belongs to the small round blue cell tumor family and presents the distinctive translocation of FET-ETS family genes (85% with EWSR1), generating gene fusions. Extraskeletal ES mainly occurs in soft tissues; the urogenital tract is rarely affected, and ureteral localization is an exceptional event with only 4 cases described in the literature. Here we report the first Italian case of primary ES of the ureter, a 24-year-old young man with lower back pain and a narrowed left ureteral lumen on CT scan. ES of the urogenital tract is an almost unique condition with a nonspecific clinical presentation and a challenging diagnosis for pathologists. We encourage awareness of these exceptional events in the differential diagnosis of ureteral lesions in young patients. Frontiers Media S.A. 2023-01-05 /pmc/articles/PMC9851605/ /pubmed/36686730 http://dx.doi.org/10.3389/fonc.2022.1070838 Text en Copyright © 2023 Valeri, Dore, Rudini, Cieri, Elefante, Saita, Bertuzzi and Colombo https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Valeri, Marina
Dore, Leocadia
Rudini, Noemi
Cieri, Miriam
Elefante, Grazia Maria
Saita, Alberto
Bertuzzi, Alexia
Colombo, Piergiuseppe
Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title_full Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title_fullStr Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title_full_unstemmed Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title_short Case report: Primary Ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
title_sort case report: primary ewing sarcoma of the ureter, an exceptional finding of unique manifestation of disease
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9851605/
https://www.ncbi.nlm.nih.gov/pubmed/36686730
http://dx.doi.org/10.3389/fonc.2022.1070838
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